Sea urchin injuries: a review and clinical approach algorithm.

Sea urchin injuries (SUIs) are among the most common marine injuries; however, there are no guidelines to dictate appropriate workup and treatment. Complications vary significantly depending on the extent of injury, site of injury and elapsed time to medical attention. Timely and appropriate management can prevent long-term morbidity despite the deceptively innocuous appearance of these injuries. We review the English literature on SUIs and offer an algorithm to aid in the management of affected patients. We found that superficially retained spines may trigger a local granulomatous inflammatory response, while spines retained near deep structures may induce sea urchin arthritis, inflammatory tenosynovitis, among other delayed complications. Therefore, we recommend immediate inactivation of pro-inflammatory compounds by hot water soaks at the time of injury, followed by extraction of all spines by a physician. Imaging is a valuable component of risk stratification to determine depth and location of spines, which guides selection of appropriate extraction technique to prevent long-term morbidity.

Solitary congenital Langerhans cell histiocytoma: A pattern of benign, spontaneous regression in patients with single lesion disease.

Langerhans cell histiocytosis (LCH) is the neoplastic proliferation of dendritic langerin-positive histiocytes manifesting as either single system unifocal, single system multifocal, or multisystem disease. The designation Hashimoto-Pritzker, or self-healing LCH, has fallen out of favor since it is impossible to predict at time of diagnosis whether the disease is truly self-remitting or capable of spreading to other organ systems. We review the English literature on solitary congenital Langerhans cell histiocytoma, draw novel conclusions from the data provided by 81 cases in the literature, and illustrate a typical presentation of the diagnosis with a previously unreported patient. Each of the patients diagnosed with solitary congenital histiocytoma experienced spontaneous resolution and had no signs of systemic disease at latest follow-up. Furthermore, we offer an analysis of the histopathological findings available from the 81 cases and our patient. Based on our study observations, we propose solitary congenital Langerhans cell histiocytoma may portend a good prognosis and represent a distinct entity. However, until further confirmation with prospective studies, we recommend clinicians continue conducting appropriate workup to rule out systemic involvement.

Enhanced cutaneous Rock2 expression as a marker of Rho Kinase pathway activation in autoimmune disease and Kohlemeier-Degos disease.

The small guanosine triphosphatase Rho and its target Rho kinase are involved in a heterogeneous spectrum of cellular activities, many of which are integral to cytoskeletal organization. Furthermore, the Rho kinases result in NF kappa beta activation and hence the induction of various pro-inflammatory cytokines including TNF-alpha, IL-1B and IL-6. ROCK2 is a downstream protein, whose expression is indicative of Rho Kinase activation. Given the diverse effects of Rho-kinase, including a potentially critical role in augmenting inflammation, ROCK2 expression was examined in biopsies of select autoimmune connective tissue diseases as compared to control diagnoses. Select cases of lupus erythematosus, dermatomyositis, autoimmune sclerodermoid disorders and Kohlmeier-Degos disease (a distinctive vasculopathy that occurs in the other aforesaid conditions but also as a forme fruste microvascular and arteriopathic syndrome) were studied. Control biopsies included normal skin and cutaneous inflammatory conditions unrelated to collagen vascular disease/autoimmune disease. We found ROCK2 expression significantly increased in biopsies of lupus erythematosus, dermatomyositis, scleroderma and Kohlmeier-Degos disease. A pattern emerged of consistent marked ROCK2 upregulation in endothelium and variable expression in inflammatory cells and epithelium. While expression was undetectable in normal skin, it was found in inflamed skin unrelated to specific autoimmune disease. The staining pattern could approach that seen in study group cases but was less pronounced and preferentially upregulated in the endothelium, with a lesser extent of staining in the epidermis and inflammatory cells. Rho kinase is a driving factor in diverse cutaneous diseases especially autoimmune disease and Kohlmeier-Degos disease. This significantly upregulated pathway defines a potential target for biologic therapy.

Denial of Illness in a Patient with Infiltrative Basal Cell Carcinoma.

A 73-year-old Caucasian man presented with a pink, pearly papule anterior to his right ear. He was a well-educated, retired stockbroker. Biopsy revealed an infiltrating basal cell carcinoma (BCC). The dermatologist repeatedly attempted to contact the patient encouraging treatment. After ignoring calls and letters, he was lost to contact with dermatology for 10 years. In the interim, the patient presented to the emergency room after discovering maggots in his ear. He subsequently consulted a head and neck surgeon but refused the recommended surgical excision. Although still operable, by this time the patient had developed significant erosion (Figure 1), nerve damage with loss of taste, facial muscle control, and hearing loss. One year after surgical consultation, he returned to dermatology due to ear discharge and pain while chewing. Multiple clinicians urged him to reconsider surgery. The patient stated that he had avoided treatment for the previous 10 years, because he had felt "stronger than the cancer." He had been convinced that cancer "can't hurt me." Despite this, he conceded that denying treatments earlier was "probably the worst decision of my life." By then the cancer was inoperable and required chemotherapy. The patient again refused treatment and later expired.

The molecular-based differentiation of Heck's disease from its mimics including oral condyloma and white sponge nevus.

Heck's disease (focal or multifocal epithelial hyperplasia) is a benign, rare condition of the skin and mucous membranes induced by human papillomavirus (HPV) infection. Other entities that can induce large papillomatous lesions that involve the mucous membranes and skin include condyloma acuminatum, which is sexually transmitted, and white sponge nevus, often due to a mutation of cytokeratin 4 or 13. Six cases diagnosed as either Heck's disease (n = 2) or white sponge nevus (n = 4) and 6 oral condyloma were compared on histologic grounds and analyzed in situ for HPV DNA, including HPVs 6,11, and 13, as well as cytokeratins 4 and 13. Each case showed marked acanthosis, and para/hyperkeratosis. More variable histologic findings included rete ridge elongation, keratinocyte degeneration, and perinuclear halos. High copy HPV 13 DNA was evident in the squamous cells towards the surface in the two cases diagnosed as Heck's disease and in two cases diagnosed as white sponge nevus on clinical grounds. HPV 6/11 was found in each of the six condyloma. Marked decrease in either cytokeratin 4 or 13 was evident in the two cases diagnosed as white sponge nevus that were HPV DNA negative. It is concluded that in situ hybridization analyses including HPVs 6, 11, and 13 as well as immunohistochemistry for cytokeratins 4 and 13 can differentiate Heck's disease from condyloma and white sponge nevus, which can be difficult to differentiate on clinical and histologic grounds.

Upper lip frenotomy for neonatal breastfeeding problems.

OBJECTIVES: Upper lip tie, without concomitant tongue tie, can prevent proper flanging of the upper lip during breastfeeding, resulting in a poor seal and suck for the infant with nipple pain and maternal dissatisfaction. Due to the lack of published studies on this subject, we report our technique and outcomes for in-office release of isolated upper lip tie. METHODS: Using CPT Code 40,806 for 'incision of labial frenulum', 22 mother-infant dyads with infant age under 60 days with breastfeeding problems and a restrictive upper lip frenum were identified. These infants underwent in-office release of upper lip tie as detailed below. Outcomes of the procedure were assessed by a telephone survey to mothers within the 4-week period post-procedure. RESULTS: 82% of mothers reported an improved latch and 73% noted increased satisfaction with breastfeeding. Lip pain, if present, resolved within 24 h for most children. Recurrence was reported by 9% of mothers; no infection or other complications occurred. CONCLUSION: Upper lip frenotomy, in properly selected infants, has favorable short-term outcomes with mild transient discomfort and a low rate of recurrence. Since our study was short-term and did not include a control group, we are unable to comment on procedure efficacy or long-term impact.

A comparison of the detection of biomarkers in infections due to low risk versus high-risk human papillomavirus types.

Adjunctive immunohistochemistry tests for human papillomavirus (HPV) infection include p16 and Ki67 as well as the more recently discovered biomarkers importin-ß, exportin-5, Mcl1, and PDL1. The purpose of this study was to compare the expression of these biomarkers in HPV infection due to the high-risk types such as HPVs 16, 18, 31, 33, 35, and 51 versus lesions that contain the low risk types HPV 2, 6 or 11. We studied 35 lesions with low risk HPV types (verruca vulgaris = 10 cases, condyloma acuminatum = 15 cases, CIN 1 with HPV 6/11 = 10 cases) and 25 CIN 1 or 2 lesions with a high-risk HPV type. The 25 high-risk positive CIN 1-2 cases had strong expression of the panel p16, Ki67, importin-ß, exportin-5, Mcl1, and PDL1 where each protein localized to the cells in the parabasal aspect of the lesion. In comparison, neither p16, importin-ß, exportin-5, Mcl1, nor PDL1 were increased in the epithelia of the lesions with the low risk HPV types; Ki67 showed variable expression. HPV viral capsid L1 protein and viral DNA were excellent markers of infection in the lesions with low risk types. Thus, p16, importin-ß, exportin-5, Mcl1, and PDL1 are not only biomarkers of high-risk HPV infection but can also differentiate such lesions from those that contain low risk HPV types. Low risk HPV infections can be best differentiated from their mimics by viral L1 capsid detection and/or HPV DNA by in situ hybridization.

Pediatric oral Epstein-Barr virus associated self-remitting CD30+ lymphoproliferative disorder: A distinct entity.

Epstein-Barr virus (EBV) has a well-known association with lymphoproliferative disorders of B and T cell origin. EBV-related B cell lymphoproliferative disorders include Hodgkin and Burkitt lymphomas, lymphomatoid granulomatosis, EBV positive diffuse large cell B cell lymphoma of the elderly, as well as B cell lymphomas associated with solid organ transplantation and methotrexate use. EBV-related T cell disorders are primarily represented by NK/T- cell lymphoma. In a subset of patients, EBV has been implicated in CD30 positive B cell lymphoproliferative disorders of the oral mucosa falling under the rubric of the mucocutaneous ulcer of the oral cavity. We previously reported on an index series of endogenous CD30 positive T cell lymphoproliferative disorder of the oral cavity resembling borderline type C lymphomatoid papulosis. The clinical manifestation of type C oral lymphomatoid papulosis is that of a recurrent self-remitting ulcer of the oral mucosa, which histologically resembles anaplastic large cell lymphoma. Such cases can be misdiagnosed as aggressive lymphoma leading to unnecessary treatment with aggressive chemotherapeutic regimens. Whereas none of the patients in our index series exhibited EBV positivity, here we discuss a very unique example of a 14-year-old girl diagnosed with EBV positive CD30 positive lymphoproliferative disorder strongly resembling the cases of intra-oral type C lymphomatoid papulosis. The patient was initially diagnosed by a senior hematopathology consultant as having EBV positive aggressive NK/T-cell lymphoma. The significance of raising physician awareness regarding pediatric oral EBV associated CD30 positive lymphoproliferative disease of the oral cavity lies in preventing inadvertent exposure to toxic chemotherapeutic agents intended for treatment of aggressive look-alikes, namely anaplastic large cell lymphoma. Additionally, we include a literature review of similar reports of pediatric intra-oral EBV positive CD30 positive T cell lymphoproliferative disease.

Oral Lymphomatoid papulosis type C: A diagnostic pitfall, often confused with T-cell lymphoma.

Eosinophilic ulcer of the oral mucosa (EUOM) is a rare, benign, self-resolving lymphoproliferative disorder, which typically presents with asymptomatic to mildly tender ulcers. Histological findings of EUOM are characterized by a polymorphic infiltrate with many eosinophils often extending into the underlying muscle. Although this entity is well documented within the dental literature, it is not well known to physicians. The pathogenesis of the condition is unclear, although reports dating back to 1997 suggest that at least a subset of EUOM represents CD30 positive lymphoproliferative disorder (CD30+ LPD). More specifically the original report and subsequent authors suggest that the patients fall on the spectrum of CD30+ LPD most reminiscent of Lymphomatoid papulosis (LyP) seen in the skin. This oral variant of LyP would be expected to have the same diverse morphologic spectrum as that seen in cutaneous LyP. We present five EUOM patients whose biopsies showed an atypical lymphocytic infiltrate most compatible with Type C LyP, a histologically unique subset of LyP, reminiscent of the biopsy findings encountered in the reported case by Ficarra and co-workers. (Ficarra, et al., 1997) In four of the five cases, the biopsies were interpreted by expert hematopathologists as an aggressive form of peripheral T cell lymphoma resulting in recommendations to administer systemic chemotherapy to four of the patients, the scheduling of one patient for induction therapy and transplantation before revision of the diagnosis, and administration of chemotherapy to one of the patients. The natural clinical course of spontaneous regression refuted the original diagnoses as a form of aggressive peripheral T cell lymphoma. Recognition of oral LyP is critical to avoid inadvertent exposure to potentially toxic chemotherapeutic regimens intended for the treatment of high grade lymphoma.